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  • AADC Aromatic L-amino acid decarboxylase
  • ACA Antibodies recognizing the adrenal cortex
  • ADCC Antibody-dependent cell-mediated cytoxicity
  • AICD Activation-induced cell death
  • AIRE Autoimmune regulator gene
  • APECED Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy
  • APS Autommune polyglandular syndrome
  • BB Bio breeding
  • BCR B-cell receptor
  • cAMP Cyclic adenosine monophosphate
  • CaSR Calcium-sensing receptor
  • CD Cluster of differentiation
  • CTLA Cytotoxic T lymphocyte antigen
  • DPT Diabetes Prevention Trial
  • FOXP3 Forkhead box P3
  • GABA Gamma-aminobutyric acid
  • GAD Glutamic acid decarboxylase
  • HLA Human leukocyte antigen
  • IA-2 Islet cell antigen-2 (tyrosine phosphatase)
  • IFNInterferon
  • IL Interleukin
  • IPEX Immunodeficiency, polyendocrinopathy, and enteropathy, x-linked
  • LFA Lymphocyte function-associated antigen
  • MHC Major histocompatibility complex
  • NALP1 NACHT leucine-rich-repeat protein 1
  • NALP5 NACHT leucine-rich-repeat protein 5
  • NK Natural killer (cells)
  • NOD Nonobese diabetic (mice)
  • SCA Steroid-producing cell antibodies
  • SCID Spontaneous combined immunodeficiency
  • TAP Transporter associated with antigen processing
  • TBI Thyrotropin-binding inhibition
  • TCR T-cell receptor
  • TD Thymus-dependent
  • Tg Thyroglobulin
  • TI Thymus-independent
  • TNF Tumor necrosis factor
  • TPO Thyroperoxidase
  • TSH Thyroid-stimulating hormone
  • TSH-R Thyrotropin receptor
  • TSI Thyroid-stimulating immunoglobulin
  • VNTR Variable number of tandem repeats

Epidemiologic analysis of a large population reported that about 1 of 30 (3.2%) people in the United States (more than 8.5 million individuals) are currently affected by autoimmune diseases. Graves disease, type 1 diabetes, pernicious anemia, rheumatoid arthritis, Hashimoto thyroiditis, and vitiligo are the most prevalent such conditions, accounting for 93% of affected individuals. A more global approach at calculating prevalence led to a corrected estimate between 7.6% and 9.4% of the world's population as affected by autoimmune diseases (2.5 in 30 people worldwide).

These autoimmune diseases have traditionally been looked upon as forming a spectrum. At one end are found organ-specific diseases with organ-specific targets. Hashimoto thyroiditis is an example in which a specific lesion affects the thyroid (lymphocytic infiltration, destruction of follicular cells) and autoantibodies are produced with absolute specificity for thyroid proteins. At the other end of the spectrum are the systemic autoimmune diseases, broadly belonging to the class of rheumatologic disorders. Systemic lupus erythematosus is an example of a disease characterized by widespread pathologic changes and a collection of autoantibodies to DNA and other nuclear constituents of all cells. Many organ-specific autoimmune diseases are autoimmune endocrinopathies. Furthermore, most endocrine glands are subject to autoimmune attack including the adrenals (autoimmune Addison disease), gonads (autoimmune oophoritis), pancreas (type 1 diabetes), pituitary (autoimmune hypophysitis), and thyroid (autoimmune thyroid disease) (Table 2–1).

Table 2–1 Some Autoimmune Endocrinopathies, Antigens, and Autoantibodies.

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